Left Orbital Mass Research Articles

A Case of Malignant Transformation of an Orbital Epidermoid Cyst to Cystic Squamous Cell Carcinoma.

Squamous cell carcinoma of the orbit is uncommon as there is no squamous epithelium in the orbit. Thus, mechanistically squamous cell carcinoma of the orbit most commonly arises from a cutaneous lesion. Although orbital epidermoid cysts are thought to have very low malignant potential, these lesions possess squamous epithelium and theoretically can undergo malignant transformation. Here we present the case of a 63-year-old woman who presented with a 3-month history of diplopia and forehead tenderness with an orbital extraconal lesion on MRI consistent with a ruptured epidermoid cyst. Six months following resection, she suddenly experienced new-onset left upper eyelid ptosis, recurrent diplopia, and left orbital pain. MRI revealed a recurrence of the left orbital mass. Left anterior orbitotomy and biopsy revealed cystic squamous cell carcinoma. This case appears to demonstrate a very rare malignant transformation of an epidermoid cyst to cystic squamous cell carcinoma.

Differential efficacy of SV-BR-1-GM in inducing intracranial metastasis regression.

e13119 Background: Breast cancer metastasis to the central nervous system (CNS) and intracranial area often leads to severe morbidity and mortality. While systemic treatments manage systemic tumors, their impact on CNS/intracranial metastases is limited. Cell based therapies like SV-BR-1-GM may overcome this limitation. SV-BR-1-GM is a GM-CSF secreting, antigen-presenting immortalized breast cancer cell line. We report its efficacy on CNS tumor regression. Methods: Post-hoc analysis of a subset of all advanced MBC patients with CNS/intracranial metastases from previous phase 1, 2, and an ongoing randomized phase 2, trials (n=7 subjects) of SV-BR-1-GM. Bria-IMT monotherapy (intradermal SV-BR-1-GM + cyclophosphamide 300 mg/m2, +interferon-alpha) was administered q3wks to 2 subjects; an immune checkpoint inhibitor (ICI) was administered with each cycle in the combination setting (n=5). Results: There were 7 patients with intracranial metastases at baseline of whom 5 had intracranial tumor responses. Of the 5 responders, median pt age = 66 (range 58-70), median prior lines = 5.5 (3-13), and median time from initial diagnosis = 7.5 years (3-10). 4/5 pts had ≥1 HLA class match with SV-BR-1-GM. 3 were HR + and 1 HER2 +. Pt A002 (ER+/PR+, HER2-) received 6/6 planned cycles, achieving an 83% reduction in breast lesions; after 3 months off therapy, imaging revealed multiple brain metastases and recurrence of right breast lesions. Following 3 additional cycles there was marked regression in both brain and breast lesions. Pt 02-003 (ER-/PR-, HER2 1+ (IHC)) received 5 cycles of SV-BR-1-GM and observed a 60% reduction (5mm -> 2mm) in a left parietal periventricular lesion, and complete resolution (CR) of a left parietal lobe. Pt 06-005 (ER+/PR-, HER2 1+) demonstrated a 56% (9mm -> 4mm) reduction in the thickness of a dural lesion overlying the left anterior temporal lobe after 6 cycles of SV-BR-1-GM + ICI. After 2 additional cycles of SV-BR-1-GM + ICI and 1 cycle of SV-BR-1-GM (ICI skipped on final cycle due to grade 2 AE), patient achieved CR of a left orbital mass observed at baseline (24mm -> 0m). Pt06-007’s (ER-/PR-, HER2 1+) CNS lesions showed a median reduction of 49% compared to baseline after 3 cycles. Pt 11-018 (ER+/PR-, HER2 +) showed standard progression (SD) at initial restaging; 14% increase in sum of diameters of intracranial metastasis. Subsequent imaging, after 3 cycles, showed significant orbital tumor reduction along with reported improvement in eye pain. Conclusions: This post-hoc analysis demonstrated promising efficacy of the Bria-IMT regimen either alone or in combination with ICIs in CNS/intracranial metastasis. The CNS/intracranial regression seen across all breast cancer subtypes among heavily pretreated patients highlights the potential of SV-BR-1-GM in managing CNS metastasis. Ongoing trials will further evaluate the efficacy of the Bria-IMT regimen in patients with CNS/intracranial metastasis. Clinical trial information: NCT03328026 .

A Pathophysiological Approach to Spontaneous Orbital Meningoceles: Case Report and Systematic Review.

Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review. A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations. A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment. The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.

Abstract CT204: Efficacy of Bria-IMT regimen in inducing CNS metastasis regression

Abstract Objectives: Breast cancer metastasis to the central nervous system (CNS) often leads to severe morbidity and mortality, and while systemic treatments manage primary tumors, their impact on CNS metastases is limited. This retrospective analysis evaluates the efficacy of Bria-IMT as a monotherapy or in combination with an immune checkpoint inhibitor (ICI) on CNS tumor regression. Methods: Post-hoc analysis of a subset of advanced MBC patients with CNS metastases from previous phase 1 and 2, and ongoing randomized phase 2 trials involving SV-BR-1-GM. SV-BR-1-GM is a GM-CSF secreting, antigen-presenting immortalized breast cancer cell line. Bria-IMT (SV-BR-1-GM ~20x106 cells, intradermally 48-72 hours after cyclophosphamide 300 mg/m2, followed by interferon-alpha at the inoculation sites 2 days later) was administered q3wks. In the combination setting, an ICI was administered with each cycle. Patients A002 and 02-003 received Bria-IMT as monotherapy. Patients 06-005 and 06-007 received Bria-IMT in combination with an ICI. Results: Median pt age of 65 (range 58-70). All pts had ≥1 HLA match with SV-BR-1-GM. Significant CNS tumor regression was observed in all pts. Pt A002 (ER+/PR+, HER2-) received 6 cycles, achieving an 83% reduction in breast lesions. ~3 months after the last cycle, imaging revealed the onset of multiple brain metastases and recurrence of right breast lesions. Following 3 additional cycles, marked measurable regression in both brain metastases and breast lesions was noted. Pt 02-003 (ER-/PR-, HER2 1+ (IHC)) received 5 cycles and observed a 60% reduction (5mm ->2mm) in a left parietal periventricular lesion, and complete resolution (CR) of a left parietal lobe lesion. Pt 06-005 (ER+/PR-, HER2 1+(IHC)) demonstrated a 56% (9mm -> 4mm) reduction in the thickness of a dural lesion overlying the left anterior temporal lobe after 6 cycles of SV-BR-1-GM + ICI (pembrolizumab). After 2 additional cycles of SV-BR-1-GM + ICI (retifanlimab) and 1 cycle of SV-BR-1-GM (ICI was skipped on final cycle due to grade 2 AE), patient achieved CR of a left orbital mass observed at baseline (24mm -> 0m). Circulating cancer-associated macrophage-like cells (CAMLs) varied throughout the treatment, with levels ranged from a baseline of 90 to 24 by Cycle 13. Pt 06-007 (ER-/PR-, HER2 1+ (IHC)) demonstrated significant reductions in CNS lesions after 3 cycles of SV-BR-1-GM + ICI (pembrolizumab): a 67% reduction in a right frontal lobe lesion, 29% in the right parietal, 30% in the right thalamus, and 67% in the right precentral gyrus lesions. CAML levels remained low throughout treatment. Conclusions: This review consolidates evidence for the efficacy of the Bria-IMT regimen in CNS metastasis regression, both alone and with ICIs. The consistent regression seen across all breast cancer subtypes among heavily pretreated patients highlights the potential of SV-BR-1-GM in managing CNS metastasis. Ongoing trials will determine the full extent. Citation Format: Sailaja Kamaraju, Blaise Bayer, Mingjin Chang, William Williams, Charles Wiseman, Giuseppe Del Priore. Efficacy of Bria-IMT regimen in inducing CNS metastasis regression [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 2 (Late-Breaking, Clinical Trial, and Invited Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(7_Suppl):Abstract nr CT204.

Primary extragonadal yolk sac tumor of the orbit: A rare case and review of literature

Germ cell tumors usually arise from primordial cells in gonads, mainly testes, and ovaries. Extragonadal involvement of orbit is very unusual. We report one case of primary yolk sac tumor of orbit in a 3-year-old male child presented as a left lateral orbital mass. Its clinical presentation may mimic many common pediatric orbital tumors causing misdiagnosis which affects ocular morbidity and mortality. Awareness about this rare entity to clinicians is helpful in the early diagnosis and proper management of young kids.

S3016 Hepatocellular Carcinoma as an Uncommon Cause of PTHrP-Mediated Hypercalcemia

Introduction: Humoral hypercalcemia of malignancy driven by PTHrP is a syndrome frequently associated with squamous cell carcinoma. We present a case of humoral hypercalcemia as a rare manifestation of hepatocellular carcinoma and describe the impact of early diagnosis on patient care. Case Description/Methods: A 67-year-old male with HCC presented to the emergency department with subacute confusion, constipation, and lower extremity weakness. Physical exam revealed tangential thinking and no asterixis. Serum calcium was 14.8 (corrected to 15.3), creatinine 1.36 (baseline 0.8), AST 141, ALT 32, and AFP 135 (all previously normal). Imaging revealed new liver lesions in multiple segments, enlarged lymph nodes in the porta hepatis, widespread lytic lesions in the spine, calvarium, and facial bones with a 3.4 cm x 2.4 cm left orbital wall mass consistent with metastatic disease. Serum calcium levels normalized with IV fluids, calcitonin, and zolendronic acid. The PTHrP was elevated at 79. Vitamin D levels, SPEP and UPEP were normal. He returned one week later with recurrent severe hypercalcemia. No further cancer treatment options were available so the patient elected for hospice services and died at home. Discussion: Malignancy is the most common cause of hypercalcemia in the inpatient setting. Malignancy-associated hypercalcemia occurs through three distinct mechanisms – humoral (PTHrP mediated), lytic bone lesions, and increased absorption due to excess 1,25 vitamin D production by the cancer. Most cases of HCC-associated hypercalcemia are due to metastatic lytic bone lesions. Less than 10% of cases are attributable to PTHrP, which is severe and often refractory. The mechanism for this is unknown but may be related to an underlying paraneoplastic syndrome and downstream metabolic derangements. Humoral hypercalcemia in patients with HCC is associated with a more advanced TNM stage and higher tumor burden. In a study of 534 patients with HCC, 6.3% had humoral hypercalcemia and had worse prognoses by Child-Pugh scores. Another study of 165 patients with HCC and PTHrP-mediated hypercalcemia found a median survival time of 15 days. Early recognition of humoral hypercalcemia in patients with HCC is essential given its association with higher mortality. Doing so can help providers expedite locoregional therapy or guide goals of care discussions with their patients.

Primary orbital melanoma arising in an atypical diffuse (plaque-like) blue naevus/melanocytosis: a case report and review of literature

BackgroundPrimary orbital melanoma is a rare disease and can occasionally develop from a pre-existing neoplasm of the blue naevus family of melanocytic lesions.Case presentationHerein we report a rare case of primary orbital melanoma arising from an unusual atypical diffuse (plaque-like) blue naevus/melanocytosis. A 27 year old man presented with mild pain and swelling of the left eye. Magnetic Resonance Imaging revealed a left lateral episcleral orbital mass and an incisional biopsy confirmed the diagnosis of malignant melanoma. Skin-sparing total left orbital exenteration was performed. Histopathological examination of the exenteration specimen revealed a primary orbital melanoma arising in a pre-existing blue naevus like melanocytosis.We demonstrate the evidence for histological progression, characterise the molecular profile of this tumour and discuss the related literature.ConclusionsThis case emphasises the importance of a meticulous clinicopathological correlation in recognising such a tumour as a primary orbital melanoma rather than a metastasis, which is managed differently.

A rapidly enlarging left medial orbital lesion

A 49-year-old man presented with a 2-month history of a rapidly enlarging left medial orbital mass. There was no local inflammation, pain, discharge, visual or nasal symptoms. On examination, there was a firm mass bulging between the left nasal bone medially and the left eye measuring 2x1cm in dimension (Fig. 1), with no overlying skin

O04 Orbital lymphoma in a 72-year-old lady with rheumatoid arthritis: an argument for rituximab

O04 Orbital lymphoma in a 72-year-old lady with rheumatoid arthritis: an argument for rituximab

Orbital melanotic neuroectodermal tumor in an infant

A 3-month-old infant patient presented with a 1-week history of a quickly progressive mass in the left eye. A hard, irregularly shaped tumor was found in the superolateral orbit. B-scan showed an uneven hypoecho and an undefined lesion. On the orbital CT, a soft tissue mass was located around the left zygomatic tubercle, involving part of the bone and lacrimal gland. The left orbital mass was removed under general anesthesia after admission. The histological and immunohistochemical examination revealed it was a melanotic neuroectodermal tumor. The infant patient was followed up for 6 months after the operation, and no recurrence was found. (Chin J Ophthalmol, 2021, 57: 372-374).

Congenital Solitary Orbital Juvenile Xanthogranuloma. Report of a Case With Challenging Clinical and Microscopic Presentation

Abstract Juvenile xanthogranuloma (JXG) is a benign cutaneous disorder. Extracutaneous mainly ophthalmic involvement is not uncommon, but congenital solitary forms are extremely rare. We report a case of a male newborn presenting for management of a left orbital mass discovered by prenatal ultrasonography. Magnetic resonance showed an orbital mass of 28-mm diameter with 5-mm intracranial extension through an enlarged superior orbital fissure. Fine-needle aspiration cytology and then an open biopsy were performed. Based on the clinical, morphological, immunohistochemical and molecular studies, a diagnosis of congenital solitary orbital JXG was made, and a wait-and-see strategy was decided. Juvenile xanthogranuloma is regarded as the most common form of non-Langerhans cell histiocytoses. Histiocytoses are rare disorders that were broadly divided into 3 categories, but recently a revised classification was proposed with categorization into 5 groups: the “L” (Langerhans) group, the “C” (cutaneous and mucocutaneous) group, the “M” (malignant) group, the “R” (Rosai-Dorfman disease; RDD) group, and the “H” (hemophagocytic lymphohistiocytosis and macrophage activation syndrome) group. In our case, clinical, histopathological, and radiological features along with molecular and immunohistochemical data are consistent with the diagnosis of JXG of the C group. In conclusion, JXG is capable of spontaneous regression, and for this, its management can be conservative.

Unilateral exophthalmos secondary to esophageal adenocarcinoma metastasis to the medial rectus muscle

Abstract A case is presented on a 51-year-old man with stage IV oesophageal adenocarcinoma. The patient was referred by the Oncology Department urgently for a same-day assessment by an ophthalmologist due to two weeks of severe unilateral exophthalmos and binocular diplopia. A comprehensive eye exam revealed the presence of an axial non-reductive exophthalmos and a limitation in left eye levoduction. A computed tomography scan was performed that showed a multiple lobed, intra- and extra-conal, heterogeneous left orbital mass, that surrounded the internal rectus muscle, compatible with metastasis, as well as another small extraconal mass at the base of the contralateral orbit. Palliative radiotherapy was then indicated. Metastases in the extraocular muscles are a very rare finding, but should be suspected in a case of unilateral exophthalmos and, if necessary, refer the patient to have the corresponding complementary tests performed.

Exoftalmos unilateral secundario a metástasis en músculo recto interno de adenocarcinoma de esófago

A case is presented on a 51 year-old man with stage IV oesophageal adenocarcinoma. The patient was referred by the Oncology Department urgently for a same-day assessment by an ophthalmologist due to two weeks of severe unilateral exophthalmos and binocular diplopia. A comprehensive eye exam revealed the presence of an axial non-reductive exophthalmos and a limitation in left eye levoduction. A computed tomography scan was performed that showed a multiple lobed, intra-and extra-conal, heterogeneous left orbital mass, that surrounded the internal rectus muscle, compatible with metastasis, as well as another small extraconal mass at the base of the contralateral orbit. Palliative radiotherapy was then indicated. Metastases in the extraocular muscles are a very rare finding, but should be suspected in a case of unilateral exophthalmos and, if necessary, refer the patient to have the corresponding complementary tests performed.

Myasthenia Gravis Associated With Orbital Marginal Zone Lymphoma

Myasthenia Gravis Associated With Orbital Marginal Zone Lymphoma

Basal Cell Carcinoma, Odontogenic Cysts, Brain and Skeletal Abnormalities (Gorlin Goltz) Syndrome in a 46-Year-Old Woman

Objective: To present the case of a 46-year-old woman with basal cell carcinoma, odontogenic cysts, brain anomalies and skeletal abnormalities.
 Method: 
 Design: Case Report
 Setting: Tertiary National University Hospital
 Patient: One
 Results: A 46-year-old woman consulted for a non-healing, necrotic left orbital ulcer that started as a skin-colored, papilla-like lesion on the upper eyelid. There were also hyperpigmented lesions with ill-defined borders over both paranasal areas. Tissue biopsies revealed basal cell carcinoma. Radiologic imaging showed cystic lesions in the mandible, straightening of cervical vertebrae and calcifications of the falx cerebri, tentorium cerebelli, pineal gland and choroid plexus. Based on established major and minor clinical and radiologic criteria, we arrived at a diagnosis of Gorlin Goltz Syndrome or Nevoid Basal Cell Carcinoma Syndrome (NBCCS). She underwent wide excision of the left orbital mass with exenteration, excision of left and right paranasal masses, left total parotidectomy with facial nerve preservation, enucleation of mandibular cyst, and cervicofacial reconstruction with skin grafts of the left orbital area and ala.
 Conclusion: NBCCS is a rare autosomal dominant disorder with a high tendency for neoplasms and developmental anomalies. Diagnosis can easily be missed if the physician is unaware of its classic but bizarre presentation. Early recognition and prompt specialist referral is very important in order to prevent complications and provide better prognosis. Patients should be reminded of the importance of follow-up as other presentations of the syndrome may manifest later in life, and family genetic screening and counseling should be undertaken.
 Keywords: Gorlin Goltz Syndrome; Nevoid Basal Cell Carcinoma Syndrome; odontogenic keratocyst

Mesenchymal chondrosarcoma of the orbit: A case report with 5 years of follow-up

ABSTRACTMesenchymal chondrosarcoma is a rare orbital tumor. Several case reports of this rare tumor have been published in the literature but only 6 cases have documented a follow up of 5 years or more. We report a case of 28 year-old female who presented with left orbital mass. Computed Tomography (CT) revealed a lobulated mass in the superior extraconal space with dense intralesional calcification. Patient underwent complete resection of the mass and histopathology was suggestive of mesenchymal chondrosarcoma. He was given adjuvant radiotherapy and there was no recurrence or metastasis at 5 years of follow-up. The case highlights that a complete resection with adjuvant radiotherapy in cases of orbital mesenchymal chondrosarcoma offers excellent prognosis.

A case report of the orbit, ocular association and the lung in granulomatosis with polyangiitis: A diagnostic challenge.

Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. A 40-year old Chinese woman was initially referred to the present institution for a progressive worsening pain, redness and gradual decrease in visual acuity in the eyes over the past 7-year period. Previous therapeutic interventions included noncompliant topical and intravenous dexamethasone for 6 years. A pre-operative examination conducted in a differing hospital to search for the presence of an orbital mass, resulted in the identification of an asymptomatic space-occupying lesion in the right middle lung, which was surgically removed in March 2015. A total of four weeks later, surgery was then applied to remove a left orbital mass, in the same hospital. A total of three months later, the patient was diagnosed with peripheral ulcerative keratitis associated with GPA, at the present institution. The corneal lesions were then treated bilaterally with cryotherapy and oral prednisone and cyclophosphamide were administered. Following surgery, the condition of the eyes appeared to be stable. A total of seven months later, the redness and pain of the right eye recurred, followed by a deep lamellar keratoplasty for the treatment of necrotizing scleritis. The condition of the two eyes was subsequently observed to be stable during the nine month follow-up. The present case study reviews various points to consider in a rare, complicated and potentially blinding case of GPA. GPA must therefore be considered in the differential diagnosis of further inflammatory conditions and tumors. Early diagnosis and an appropriate interdisciplinary approach to management, are required to decrease recurrence and morbidity in patients with GPA-mediated inflammatory ocular disease.

Unilateral Exophthalmos Due To Metastasis Of Poorly Differentiated Thyroid Carcinoma To The Left Sphenoid Wing With Intra-Orbital Extension

ABSTRACT Objective: In contrast to well-differentiated and anaplastic thyroid carcinomas, metastasis patterns are not well-known for poorly differentiated thyroid carcinoma (PDTC). We report an unusual case of PDTC initially presenting as unilateral exophthalmos. Methods: Clinical, laboratory, imaging, and pathology data are presented. Results: A 55-year-old woman without significant medical history presented to an outside hospital complaining of a “bulging” left eye for several weeks. Computerized tomography–guided excisional biopsy of a left orbital mass revealed poorly differentiated insular thyroid carcinoma (positive for cytokeratins, thyroid transcription factor-1, and thyroglobulin; negative for monoclonal carcinoembryonic antigen, S100, chromogranin, and synaptophysin) with a Ki-67 index of 5 to 10%. In the midst of outpatient management, including treatment with dexamethasone, she presented to our tertiary center in diabetic ketoacidosis. Physical examination demonstrated unilateral left-sided ex...

Unilateral orbital mass as an unusual presentation of IgG4-related disease

IgG4-related diseases are a recently recognized systemic syndrome characterized by mass-forming lesions, in mainly exocrine tissue, that consist of lymphoplasmacytic infiltrates and sclerosis, which may mimic malignant neoplasm due to clinical and imaging features. We report an unusual case of a 62-year-old woman who presented with a left orbital mass, which histologically revealed to be an IgG4-sclerosing disease.

Sarcoidosis. Debut as orbital pseudotumour

Case report78-Year-old female patient being investigated for a left orbital pseudotumour of 17 days onset. She had proptosis, pain, with no limitation of eye movements, periorbital edema, and upper eyelid ptosis, compatible with an orbital pseudotumour. The MRI showed an upper outer left orbital mass adjacent to the side of eyeball and an increase in soft tissue at the level of lacrimal gland. The histopathology study of an eyelid lesion demonstrated the presence of non-caseating granulomas. DiscussionA definitive diagnosis of orbital sarcoidosis was made. Oral treatment with corticosteroids and methotrexate achieved gradual control of the disease.